Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India.
Hepatol Int. 2018 Feb;12(Suppl 1):148-167. doi: 10.1007/s12072-018-9844-3. Epub 2018 Feb 20.
Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations.
The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO.
IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions. While the level of block in IPH is presinusoidal, in EHPVO it is at prehepatic level. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Contrastingly in EHPVO, prothrombotic disorders and local factors around the portal vein are the incriminating factors. Diagnosis is often clinical, supported by simple radiological tools. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority of patients in both the disorders. Surgical shunts revert the complications secondary to PHT. Meso-Rex shunt has become the standard surgery in children with EHPVO.
This review gives a detailed summary of these two vascular conditions of liver-IPH and EHPVO. Further research is needed to understand the pathogenesis and natural history of these disorders.
特发性门静脉高压症（IPH）和肝外门静脉阻塞（EHPVO）是非肝硬化性门静脉高压症（PHT）的非肝血管原因。静脉曲张出血和脾肿大是最常见的表现。
本综述旨在提供关于 IPH 和 EHPVO 的病因发病机制、临床特征、诊断、自然史和治疗的现有文献。
IPH 和 EHPVO 的特点均为正常肝静脉压力梯度、中等至大量脾肿大伴保留肝脏合成功能。虽然 IPH 的阻塞程度为窦前性，而 EHPVO 的阻塞程度为肝前性。在 IPH 中，感染、自身免疫、药物、免疫缺陷和血栓形成状态可能是病因。相反，在 EHPVO 中，血栓形成紊乱和门静脉周围的局部因素是罪魁祸首。诊断通常是临床的，辅以简单的影像学工具。自然史由静脉曲张出血和肿大脾脏相关症状定义。生长发育迟缓、门静脉胆病和轻微肝性脑病是 EHPVO 的其他关注点。内镜监测的长期生存情况良好，但在这两种疾病中，少数患者会出现实质消失导致失代偿。手术分流术可逆转 PHT 引起的并发症。在 EHPVO 患儿中，Meso-Rex 分流术已成为标准手术。
本综述详细总结了这两种肝脏血管疾病-IPH 和 EHPVO。需要进一步研究来了解这些疾病的发病机制和自然史。
